ANCA may be a pathogenetic force, but to date, support for this contention remains indirect. Active immunization with antigen or passive transfer of ANCA has not reproduced small vessel vasculitis (SVV). It is more than likely that if ANCA are pathogenetic, they are involved as one of many simultaneously occurring mechanisms acting in concert with other synergistic inflammatory mediators of disease. These include not only environmental factors such as infection or environmental toxins such as silica, but also genetic factors that are only now being described. The paradigm for this autoimmune process must include several events that occur simultaneously or sequentially, including ANCA, leukocyte activation and injured endothelium.
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